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Case Report DOI: 10.18231/2394-6792.2018.0029

Squamous in a pre-existing adult mature Sacro-Coccygeal : A rare case with review of literature

Ranjan Agrawal1,*, Jagdamba Sharan2, Parbodh Kumar3

1Professor, Dept. of & Surgery, Rohilkhand Medical College & Hospital, Bareilly, Uttar Pradesh, India

*Corresponding Author: Email: [email protected]

Abstract We report a case of with squamous cell carcinomatous transformation in a 40-year-old lady. This is an infrequent location for teratoma in adults and malignant transformation has rarely been described. The uniqueness of the present case was the presence of congenital sacrococcygeal teratoma presenting in adulthood with malignant transformation.

Keywords: Malignant transformation, Sacrococcygeal, , Teratoma.

Received: 04th July, 2017 Accepted:

Introduction non-tender, fluctuation present, non-reducible or compressible with no abnormal pulsations. The are found with decreasing skin over the swelling was tethered in the center frequency in the gonads (ovaries and testis), but at the periphery it was free from the swelling. mediastinum, retroperitoneal space, Another swelling was present just below the sacrococcygeal region, pineal and other coccyx about 8 cm × 6cm in size and intracranial locations.1,2 Sacrococcygeal communicating with the swelling in the sacral teratomas are congenital tumour that develop region as on pressing either of the swelling the embryologically from multipotent cells and other swelling increased in size due to movement enlarge as pre and post sacral mass. As per their of fluid (cross fluctuation sign positive). degree of maturation, they are classified as Routine tests were within normal mature, immature, and malignant. We present an limits. The pus which was drained out showed unusual case of congenital sacrococcygeal plenty of pus cells and culture isolated coagulase teratoma in a 40 year old female, later negative staphylococci sensitive to most undergoing malignant transformation. antibiotics. No acid fast bacilli were present. USG abdomen did not show any significant Case Report abnormality except for incidental gall stones. MRI showed a large lobulated multiseptated A 40 year old female presented with an heterogenously hyperintense altered signal increasing swelling in the lower back since last 3 intensity mass lesion at posterior aspect of months. The swelling was present since birth, sacrococygeal region, with no evidence of but had increased in size recently. There was no osseous destruction, intra- pelvic extension or other significant history. Her menstrual history inflammatory changes in the peripheral was normal with regular cycles of normal myofascial plane. Under epidural anaesthesia duration and flow. Patient was married, had 6 through a vertical slightly curved incision the children all born by normal vaginal delivery at tumour was explored. Skin flaps were raised and home. She recently developed mild fever and the tumour was dissected free from the skin and sudden significant increase in the size of the adjacent tissues. There were three extensions of swelling 3 months back for which a local the tumour, one in the sacral region, second in practitioner was consulted who aspirated copious the coccygeal region and third towards the left amount of purulent fluid (300 – 400 ml) from the lateral side away from the midline reaching up to swelling. After two-and half months the swelling the skin. All the three extensions were recurred for which she was referred to this communicating with each other. Grossly, the tertiary care hospital. tumour measured more than 25×18 cm in size On local examination a swelling was noted (Fig.1). in the sacral region measuring 12 cm× 15cm in The tumour was adherent to the sacrum size covered with dark coloured skin. The local posteriorly at the site of the junction of the three temperature was not raised. The swelling was extensions which was fulgurated, two drains Indian Journal of Pathology and , January-March, 2018;5(1):161-163 161 Ranjan Agrawal et al. Squamous cell carcinoma in a pre-existing adult mature Sacro-Coccygeal…

placed and the wound closed. The extragonadal. In children, sacrococcygeal histopathological examination of the resected teratomas comprise of 40% of all GCTs and up specimen suggested mature teratoma with areas to 78% of all extragonadal GCTs. of squamous cell carcinoma changes i.e., Sacrococcygeal teratoma is the most common Teratoma with malignant transformation (Figs. 2 solid in neonates, with an estimated and 3). The malignant squamous part showed prevalence of 1 in 35 000–40 000 live births with positivity with (Fig. 2b). Post- female to male ratio 4:1.1 Adult sacrococcygeal operative period was uneventful and the patient teratoma is extremely rare with an incidence of was discharged with an advice for regular follow 1:87,000 and a female to male ratio of 10:1. up. Most common are benign, also called mature teratomas. Sacrococcygeal teratomas with malignant elements generally are not seen in infants. is very rare in adult sacrococcygeal teratomas and if present arises from the embryonic tissue. In the present case malignant change was noted in the squamous . Squamous cell carcinoma has been reported in only a single case.3 A total of 92 cases sacrococcygeal teratomas in adults, with only 20 as malignant or with malignant 1,3-5 transformation are available in the literature. Fig.1: Gross appearance of the resected Most adult sacrococcygeal teratomas are cystic specimen and benign and only 1-2% are malignant. The risk of malignancy increases with age.6 Mature Sacrococcygeal teratomas can undergo dysplastic change and frank malignant degeneration. Sacrococcygeal teratomas are thought to originate from multipotent cells in Henson’s node, which migrates caudally to rest in the coccyx. All Sacrococcygeal teratomas involve the coccyx. They may grow postero-inferiorly into the gluteal area and/or antero-superiorly into the abdomino-pelvic cavity.1,7 Most adult Sacrococcygeal teratomas are intra pelvic.4 However, there was no intra pelvic extension in the present case. Fig. 2: Microphotograph showing (a) areas of Teratomas often are comprised of cells that normal squamous lining; (b) malignant represent all three germ cell layers. They have squamous Component; and (c) malignant solid, cystic or mixed components. Unlike squamous component positive for Cytokeratin teratomas in other locations, sacrococcygeal (40X) teratomas often do not have a capsule or pseudocapsule, which contributes to the difficulty in achieving a complete resection. Exclusively pre-sacral tumours present later than those with an external component, and have a higher prevalence of malignant transformation Solid tumours are more likely to be malignant. Necrosis, poor line of demarcation of the

Fig. 3: Microphotograph showing other adjacent soft tissue planes, of adjacent components of teratoms – adipocytes, blood structures, destruction of sacrum, regional lymphadenopathy and distant metastases are vessels, fibrous tissue and neural tissue. (40 X) 1,4 signs suggestive of malignancy. Discussion Malignant teratomas, in addition to mature and/or embryonic tissues, have frankly malignant Germ cell tumours (GCTs) that arise outside tissue of germ cell origin, such as the testes or the ovaries are classified as and choriocarcinoma. Tumours containing malignant, non-germ cell elements, including Indian Journal of Pathology and Oncology, January-March, 2018;5(1):161-163 162 Ranjan Agrawal et al. Squamous cell carcinoma in a pre-existing adult mature Sacro-Coccygeal…

and squamous cell carcinoma, 3. Chen KT, Squamous cell carcinoma arising in are referred to as teratoma with malignant sacrococcygeal teratoma. Arch Pathol Lab Med transformation. Patients with either a malignant 1980;104 (6):336. PMID: 6892874 4. B. Kostas, G. Zisis, T. Christos, K. Isaak, G. teratoma or a benign teratoma with malignant Vrakas, X. Vrakas: Sacrococcygeal teratoma in transformation have a considerable increase in adults: Report Of A Case And Literature mortality. Patients with Sacrococcygeal Review. The Internet Journal of Surgery. teratomas may be asymptomatic on initial 2007;11(2). DOI: 10.5580/fe3 presentation or may manifest a variety of 5. Robin NH, Grace K, DeSousa TG, McDonald- McGinn D, Zackai EH. New finding of Schinzel- symptoms that are not indicative of either benign Giedion syndrome: a case with malignant or malignant nature. sacrococcygeal teratoma. Am J Med Genet Imaging features alone do not allow definite 1993;47:852-6. differentiation between benign and teratomas 6. Al-Essa AA, Malik TA, Baghdadi MK, El Tayeb with malignant transformation. Tumour markers, AA. Adult sacrococcygeal teratomas.Saudi Med such as alpha-fetoprotein, carcino-embryonic J. 2004 25(3):367-9. PMID: 15048179 7. Audet IM, Goldhahn RT Jr, Dent TL. Adult antigen and human chorionic gonadotropin, are sacrococcygeal teratomas. Am Surg 2000;66.61- not helpful in differentiating between benign and 5. malignant lesions.6 8. Lopes A, Pearson S, Roberts J, et al. Case report: Surgical treatment of sacrococcygeal immature presacral teratoma in an adult female. teratomas is complete excision, inclusive of Gynecol Oncol 1990;38:135–7. coccyx and may be sacrum if involved. Coccyx may contain a nidus of pleuripotent cells that increases the recurrence rates to 37% when not excised.1,2,6 Most recurrences may be due to retained tissue, spillage of contents during dissection or due to residual tissue densely adherent to the vital structures left behind at initial operation. Malignant tumours have a greater likelihood of recurrence and the incidence of malignancy is higher in recurrent than in primary tumours. [6] If complete resection is accomplished, benign teratomas have a good prognosis. Since, malignant transformation is rare there has been no standard recommendation for the use of or radiotherapy and the best treatment plan is individualised multimodality therapy.1,8 Complete resection was not possible in this case and part of the tumour wall adherent with the sacrum was fulgurated with an advice for radiotherapy on follow-up.

Conclusion

Although a rare tumour in adults, sacrococcygeal teratoma should be considered in the differential diagnosis of patients with occult or a palpable pelvic mass.

References

1. Monteiro M, Cunha TM, Catarino A, Tome V. Sacrococcygeal teratoma with malignant transformation in an adult female: CT and MRI findings. Br J Radiol 2002;75:620-23. 2. Ng EW, Porcu P, Loehrer P. Sacrococcygeal teratoma in adults: case reports and a review of the literature. 1999;86[7]:1198-202.

Indian Journal of Pathology and Oncology, January-March, 2018;5(1):161-163 163